An aphasic syndrome in children

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					                                                                                                ORIGINAL ARTICLE

An        aphasic syndrome in children
W.   McKinney,      m.d.   and    Douglas    A.   McGreal,     m.d., f.r.c.p.,   f.r.c.p.[c], Toronto
Summary: The paper presents case reports of nine children,              Case   reports
all of whom had severe disturbances of language function
of abrupt or gradual onset. Seizures occurred in seven of               Case 1
the children and all nine had EEG abnormalities.                           A 4-year-old white girl born after a normal pregnancy and
There was no apparent correlation between the seizures,                 labour weighed 2950 g at birth. She developed normally in
the EEG changes and the aphasia. One patient had a brain                all respects including speech until about the age of 3 years,
biopsy performed at another hospital and the tissue was                 when the mother thought the child was ignoring verbal instruc-
said to be normal. The cause of the abnormal language                   tions. An otolaryngologist consulted at this time said that the
function is unknown. Five of the children failed to                     child's hearing was normal. By the age of 4 years her speech
acquire normal speech subsequently. Three of the four                   was above average, according to her parents, and she was
children who made a complete recovery were treated                      considered by her kindergarten teacher to be very bright.
with steroids.                                                             Suddenly one month later her speech became reduced to
                                                                        monosyllables and was difficult to understand. She seemed
                                                                        to understand gestures and pictures but not spoken commands.
Resume: Un syndrome aphasique de l'enfant                               She had no loss of motor skills. She had never had seizures
   Cet article presente neuf enfants qui souffraient tous               or other neurological symptoms and there was no family
d'une alteration de la capacite de parler dont le debut                 history of speech problems, mental retardation or neurological
avait ete brusque ou progressif. Sept des enfants avaient eu            illness.
des convulsions et chez les neuf, on notait des anomalies de               On examination she appeared to be physically normal but
I'EEG. Apparemment, il n'y avait pas de correlation                     had both receptive and expressive aphasia. Hearing and visual
entre les convulsions, les modifications de I'EEG et I'aphasie.         comprehension were normal. During an EEG recording she
Une biopsie cerebrale avait ete pratiquee chez un enfant                complained of a left-sided headache and had a generalized
                                                                        seizure during which the EEG showed diffuse seizure activity,
dans un autre hopital d'ou il ressortait que le tissu                   more prominent on the left. She was given diphenylhydantoin
etait normal. La cause profonde du trouble du langage                   and, in addition, daily injections of ACTH. After five days
reste inconnue. Cinq des enfants n'ont pu acquerir                      of steroid therapy the EEG was repeated and it showed an
un langage normal par la suite. Trois des quatre enfants                excess of slow activity, together with seizure activity, in both
qui ont eu une guerison complete avaient ete traites par                postcentral regions, but more prominent on the left. The
des corticoides.                                                        cerebrospinal fluid was normal and a pneumoencephalogram
                                                                        showed only minimal dilatation of the left temporal horn. She
                                                                        was receiving prednisone orally when discharged and this was
In 1957 Landau and Kleffner1 of the Central Institute                   continued for two weeks. Six weeks later her speech was
for the Deaf, St. Louis, a referral centre for hearing                  normal, and no further seizures have occurred.
problems, described six children in whom language prob¬                 Case 2
lems developed in association with a seizure disorder. The
seizures were readily controlled and the language difficulty               A white boy had severe neonatal jaundice. He was born
                                                                        at term, the second child of an Rh-negative mother, and
slowly returned to normal. Worster-Drought's2 14 patients               weighed 2720 g at birth. He received an exchange transfusion
reported in 1971 also had seizures and abnormal electro¬                and additional simple transfusions in the first weeks of life.
encephalograms and some did not recover normal speech.                     When he was 3 years old he had a generalized seizure and
Gascon et aP in 1973 described three more patients with                 was brought to hospital where he appeared bright and alert.
language disorder, seizures and incomplete recovery of                  No signs of neurological abnormality were found. An EEG
speech. The purpose of this communication is to outline                 reported to show "paroxysmal dysrhythmia" is not available
experience with this syndrome at The Hospital for Sick                  for review. He was given anticonvulsant drugs. He had been
Children, Toronto. Nine case reports are presented.                     late in walking and was only just beginning to say a few
                                                                        words. Because his speech development continued to be delayed
                                                                        he was assessed psychologically when he was 5 years old and
From the Department of Paediatrics, The Hospital for Sick Children,     the psychologist commented that the mother's description of
Toronto                                                                 the boy as "a little mimic" seemed most appropriate. He
Reprint requests to: Dr. D. A. McGreal,                                 could understand gestured directions but did not understand
555 University Ave., Toronto, Ont. M5G Hospital
                                                for Sick Children,
                                        1X8                             spoken instructions. His hearing was normal.
                                                                                 CMA JOURNAL/MARCH 16, 1974/VOL. 110 637
   The patient was next seen at age 21 years. He had had no          to be transient and recurrent.    There   was no   family history
seizures or anticonvulsant medication for many years. His            of  speech problems, mental retardation or seizures.
speech is limited to about 100 garbled words. Hearing is                On admission to hospital at 5 years of age he seemed
normal and he is said to have a "terrific memory" and al¬            physically normal but he had a receptive and expressive
though he cannot read he understands gestures and pictures           aphasia. His EEG showed slow and sharp waves in the left
and draws extremely well. He works successfully as a short-          postcentral head region. A pneumoencephalogram was normal,
order cook in a restaurant where he is able to fill the customers'   as was the cerebrospinal fluid. Even though no definite seizures
orders from the slips left at his counter by the waitresses. His     had occurred diphenylhydantoin was prescribed.
parents say that except for his "retarded speech" he is normal.         When seen a few months later his speech had improved
                                                                     and he had a useful vocabulary approximating that of a
Case 3                                                               3-year-old, and he appeared to hear normally. He was placed
   A white boy was first examined at the age of 5 years, 5           in a class for aphasic children.
months. He had been born spontaneously after a normal                   At 6 years of age he appeared to be making satisfactory
pregnancy, was two weeks premature and weighed 2300 g                progress according to his parents and teachers, but whenever
at birth. He spoke his first word at about 1 year of age but         he developed a respiratory tract infection his ability to com-
did not progress beyond single, one-syllable words until about       municate deteriorated.
3 years of age. During his fourth year he spoke short sentences         A year later a left frontotemporal craniotomy and temporal
but then regressed, and at the time of admission at 5 years          lobectomy were performed at another hospital. The biopsy
of age he was again speaking only single words. A few                material showed no significant morphological changes. Since
days after beginning school he had a generalized seizure. An         this operation he has had no more "ups and downs". He never
older sibling, the mother and the maternal grandmother had           has had seizures. He still receives diphenylhydantoin regularly.
all had seizures.                                                    His IQ is normal, he has almost normal speech and appears
   The boy appeared to be retarded and distractible. Although        to understand speech "most of the time". He has difficulty
his hearing was normal his ability to understand speech was          reading but understands gestures and pictures and has no
questionable and his ability to express himself was obviously        motor   deficits.
deficient. His right arm, leg and face were smaller than the         Case 6
left despite the fact that he was right-handed, right-footed and
left-eyed. Psychological testing was performed with difficulty          A 5-year-old white boy was admitted because one year
and he was given an IQ rating of 74. His EEG was diffusely           previously he had suddenly stopped speaking and appeared
abnormal and has remained so, usually the left hemisphere            to have hearing difficulty. There was some improvement in
being more severely affected. He was given diphenylhydantoin.        his hearing after his tonsils and adenoids were removed, but
   Numerous seizures occurred in the following years, usually        his speech did not return.
during sleep, but they are becoming less frequent. The most             He was born spontaneously after an uneventful pregnancy
recent one, at age 13 years, occurred after his anticonvulsant       and weighed 3860 g at birth. Motor skills developed normally
medication had been omitted for several days. He remains             and he was able to say single words with meaning by 12
generally slow, has particular difficulty expressing himself, but    months of age and to put two or three words together by
his visual comprehension is good.                                    20 months of age.
                                                                        On admission he appeared to have no neurological defect.
Case 4                                                               He was the only left-handed member of a right-handed family.
   A 13-year-old white girl appeared well but suddenly she           The boy had never had any seizures and the family history
                                                                     was negative for speech problems, seizures, mental retardation
had two syncopal episodes on the same day, each lasting two          and neurological illness. His hearing and IQ were normal.
to three minutes and followed by a brief period of confusion.        His EEG showed much seizure activity during sleep, the
One week later she began having left-sided seizures and de¬          disturbance being more prominent in the temporal regions
veloped slurred speech. She was given diphenylhydantoin and          bilaterally. Anticonvulsants were prescribed.
four days later was found in bed with her eyes deviated to              Seven years later he is attending a school for aphasic children
the left and both hands twitching. She had difficulty speaking.      and is graded above average on IQ testing. He has some
She is right-handed, right-footed and right-eyed. Several similar    articulation problems and some difficulty in understanding
episodes preceded her admission to hospital a week later.            speech but reading comprehension is excellent and he con-
   She had been born spontaneously after a normal pregnancy          tinues to be seizure-free.
and weighed 2860 g. Her general development had been
normal and she was an excellent student at school. There             Case 7
was no family history of neurological disease.
   On admission she was found to have mild left-sided in-               A 4-year-old white boy began having night-time seizures in
coordination. Her speech impairment was striking; word-              which he would cry out and his parents would find him lying
finding and formulation were difficult. She had verbal apraxia.      with his limbs extended and stiff. These spells lasted up to
Her auditory comprehension, tested with some difficulty, ap¬         five minutes and were repeated several times a night. A
peared adequate for simple concrete ideas and simple com¬            month later he began to have seizures during the day and
mands. Hearing was normal. The EEG showed a diffuse                  then, one week before admission, he became unable to speak.
disturbance of the right hemisphere, maximal in the mid-head            He had been delivered normally after an uncomplicated
region. A pneumoencephalogram showed only minimal dilata¬            pregnancy, weighing 3520 g, but was said to be two weeks
tion of the anterior portion of the left lateral ventricle. A left   postmature. His subsequent development was normal in all
carotid angiogram was normal as was the cerebrospinal fluid.         respects.
  She was given diphenylhydantoin and phenobarbital and                 On examination he had no physical abnormalities but had
had no further seizures. Her speech was slow to improve and          receptive and expressive aphasia. His EEG showed an excess
three months later she was given a course of ACTH. Rapid             of diffuse paroxysmal slow activity. A pneumoencephalogram
improvement then occurred and she has made a complete                showed minimal dilatation of the lateral ventricles and the
recovery and remains well without medication.                        cerebrospinal fluid was normal. Anticonvulsants were prescribed.
                                                                        Three months later his family took him to Italy where
Case 5                                                               he was treated with ACTH, penicillin, vitamin C and gamma
   A 5-year-old white boy was admitted because of "recurrent         globulin. The medications were continued for a few weeks
loss of hearing". He had been born spontaneously at term             and then stopped by the parents because the boy seemed
after an uneventful pregnancy and weighed 4050 g. He appeared        normal to them. No relapse has occurred in the year and a
to develop normally but at 4 years of age his tonsils and
                                                                     half since recovery.
adenoids were removed because he seemed to have "difficulty          Case 8
hearing". Improvement after the operation was not sustained
and three months later his ability to hear had again apparently        A white girl was born after a normal pregnancy and weighed
declined. A few months later his speech regressed. An IQ             3400 g at birth. The delivery was by forceps after internal
at this time was said to be 116. His hearing difficulty continued    rotation but no problems were encountered in the newborn
638 CMA JOURNAL/MARCH 16, 1974/VOL. 110
period and she developed normally except that she was slow               respects before the language problem became evident.
in speaking. She seemed to have difficulty understanding and             Others seemed to have difficulty from an early age,
talking and at times would not attempt to talk at all. The               perhaps implying a different underlying mechanism,
parents felt that this reaction was a behavioural one. A                 but relapses and remissions occurred in some patients
maternal aunt had epilepsy.                                              in both groups. Clearly, if the pathological process,
   On examination at 6 years of age the girl appeared physically
normal, intelligent and able to hear. An EEG showed an                   whatever its nature, becomes operative before the child
active seizure focus in the left temporoparietal region. She             has developed any or much speech the problem might
was given anticonvulsant drugs.                                          be regarded as one of "developmental dysphasia".
   After discharge from hospital she received regular speech       2.    Seizures, when they occurred, were of minor concern
therapy but progress was slow for the next year. Suddenly                and seizure control was relatively easy. Two patients
speech deteriorated. Two months later she was admitted                   had no seizures. The pattern of the seizures and their
because of a right-sided seizure lasting 30 minutes. The                 temporal relation to the language disorder has varied
examination again showed no abnormalities apart from the                 from case to case and it is difficult to maintain that
aphasia. Her EEG was unchanged. Anticonvulsant medication
was continued but was finally stopped after six years without           the seizures have caused the dysphasia, rather, that
seizures.                                                               both symptoms reflect an underlying cerebral dysfunc-
   Now, at age 15, her speech is normal and she is doing well           tion. Similarly, the type of EEG abnormality does nqt
in school.                                                              help in trying to determine etiology.
Case 9                                                             3.   Examination of the specimen of brain tissue removed
                                                                        from one patient at another hospital showed no ab-
   A 12-year-old boy, a product of a normal term pregnancy,             normalities, but the fluctuating course in some patients
had weighed 2720 g at birth. He had no problems in the                  suggests an inflammatory mechanism. Recurrent ence-
newborn period but became a slow learner. He sat alone                  phalomyelitis has been described5 and it seemed that
at 5 months, walked alone at 1 year and spoke single words              in these patients infection was a predisposing factor.
with meaning at 11 months, but was 3 years old before he                Examination of the cerebrospinal fluid in some of the
put two or three words together. He was in a special class
for slow learners.                                                      present patients showed normal cell counts and protein
   Three years earlier, at age 9 years, he was found to have            values. However, the apparent good response in three
diabetes after a two-week history of polydipsia and polyuria.           patients to the use of ACTH would justify more in-
He was admitted to hospital at that time in ketoacidosis and            tensive investigation of the CSF in future patients in
discharged on lente insulin and a diabetic diet.                        order to try to document an inflammatory cause. The
   At 10 years of age, he had a grand mal seizure which was             parents of some patients felt that upper respiratory tract
followed by a receptive and expressive aphasia. His diabetes            infections preceded exacerbations, an observation im-
had been reasonably well controlled and his blood sugar                 possible to assess retrospectively.
at the time of the seizure was normal. A maternal aunt had
epilepsy and an older brother had seizures and was mentally        4.   One patient, Case 4, is right-handed, right-footed and
retarded. The patient's hearing was normal and there were               right-eyed. Her seizures and physical findings were
no neurological deficits. His initial EEG was diffusely abnormal        left-sided and the EEG disturbance predominantly of
with a generalized excess of slow activity which was maximal            the right hemisphere. This might indicate that she
over the left hemisphere and in the temporal and parietal               is one of the rare right-handed individuals in whom
regions. No seizure activity was seen. Carotid arteriograms             speech localization is in the right hemisphere, or alterna-
were normal. An air encephalogram showed slightly dilated               tively that there was involvement of the left hemisphere
ventricles. His IQ rating was between 50 and 60 with many               as well. It may be that the language disturbance results
perseverative errors.                                                   from involvement of very small areas of cortex of
   Now, at 12 years of age, he speaks in sentences of reason-
able length and complexity. Several seizures occurred in the            the dominant, or both, hemispheres though if this were
first few months after the first one but he has remained                so there should be more children with aphasia following
well controlled on diphenylhydantoin and has been seizure-free          head trauma or inflammation.
for the past year. His EEG remains poorly organized and            5.   Treatment is empirical and anticonvulsants have been
shows a seizure focus in the left anterior temporal region.             prescribed for the seizures. Because of the superficial
His verbal IQ is 62 but his performance IQ is 85 and he                 resemblance to epilepsia partialis continuans - which
has returned to his original opportunity class in school.               may be due to focal encephalitis - and because the
Discussion                                                              prognosis for full recovery of language function is
                                                                        poor, the use of ACTH or steroids is justified. Each of
   It is well recognized that aphasia can occur in children,            the three patients in this series who received this
as in adults, in association with injuries to the brain,                therapy has recovered fully.
tumours, vascular changes, infections, convulsions, etc.4
However, from a consideration of the patients reported here        References
and elsewhere 13 there emerges a clinical picture of a             1.   LANDAU WM, KLEFFNER FR: Syndrome of acquired aphasia with
child with a severe disturbance of language function, the               convulsive disorder in children. Neurology(Minneap)
onset of which may be abrupt or gradual, recovery partial          2.   WoRsTER-DRoUGHT C: An unusual form of acquired7: aphasia1957
                                                                        children. Dev Med Child Neurol 13: 563, 1971
or complete, and for which no cause is apparent. Ab-               3.   GASCON G, VICTOR D, LoMBROSO CT, et al: Language disorder, con-
                                                                              disorder and electroencephalographic abnormalities. Acquired
normalities in the EEG are the other common feature.                  syndrome in children. Arch Neurol 28:
The following observations may be made.                            4. ALAJOUANINE T, LHERMITrE F: Acquired165, 1973 in children. Brain
                                                                      88: 653, 196:5
1. Some of the children developed quite normally in all            5. ALCocx NS, HOFFMAN HL: Recurrent encephalomyelitis in childhood.
                                                                      Arch Dis Child 37: 40, 1962

                                                                            CMA JOURNAL/MARCH 16, 1974/VOL. 110 639

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